Symptomatic hepatic disease in cystic fibrosis: incidence, course, and outcome of portal systemic hunting.

Fifteen (2.2%) of 693 patients with cystic fibrosis seen over an 18- 
year period developed clinical hepatic disease. In 13 patients all symptoms 
were secondary to portal hypertension. Ten had hypersplenism and 6 had 
variceal bleeding, including 3 who developed both conditions. All 5 patients 
who survived the initial episode of gastrointestinal bleeding underwent portal systemic 
shunting. A shunting procedure also was performed on 1 patients with 
hypersplenism but no variceal bleeding. No subsequent deterioration of intellectual function 
occurred in either the shunted or unshunted patients. Only 1 of 
the shunted patients showed progression of hepatic disease after surgery. These 
results suggest that portal systemic shunting is useful in the treatment 
of bleeding esophageal varices in cystic fibrosis. A sweat test to 
rule out cystic fibrosis should be included in the evaluation of 
any teenage or young adult patient with unexplained portal hypertension. 